Varied presentations of congenital ocular synkinesis: do they all fit congenital cranial dysinnervation disorder spectrum? / Apresentações variadas de sincinesias oculares congênitas: todas elas se enquadram ao espectro das desordens congênitas da desnervação craniana?

ABSTRACT Purpose: Synkinesis results from nerve miswirings and causes aberrant movements of the affected muscles. We present a series of cases of rare congenital ocular synkinesis involving the extraocular muscles and the levator palpebrae superioris and speculate the possibility of classifying these entities in the spectrum of congenital cranial dysinnervation disorder. Methods: Records of patients with the diagnosis of congenital ocular synkinesis were analyzed retrospectively. We analyzed the sex, laterality, and complete features of the ocular motility of each patient. Results: Nine patients with congenital ocular synkinesis were included. A slight predominance of women was noted. In terms of laterality, four patients had only the right eye involved, four had only the left eye, and one had both eyes involved. Notably, 55.5% were orthotropic in the primary position. The third, fourth, and sixth cranial nerves were involved in the miswiring in 100%, 44.4%, and 11.1% of the cases, respectively. Conclusions: Congenital synkinesis might present in a very eclectic and uncommon fashion. The aberrant innervation in these cases classifies them into the group of congenital cranial dysinnervation disorders.

RESUMO Objetivo: Sincinesias são resultado de inervações anômalas e ocasionam movimentos aberrantes dos músculos envolvidos. Apresentamos uma série com casos raros de sincinesias oculares congênitas dos músculos extraoculares e do levantador da pálpebra superior e especulamos a possibilidade de classificá-las dentro do espectro das desordens congênitas da desnervação cranianana. Métodos: Prontuários de pacientes com diagnóstico de sincinesia ocular congênita foram estudados retrospectivamente. Analisamos sexo, lateralidade e as características completas do exame de motilidade de cada paciente. Resultados: Nove pacientes com sincinesias oculares congênitas foram incluídos. Houve discreta predominância no sexo feminino. Em termos de lateralidade, o olho direito foi o único envolvido em 4 casos, o olho esquerdo também em 4 casos e 1 caso apresentou acometimento bilateral. 55,5% dos pacientes eram ortotrópicos na posição primária. Os III, VI e IV nervos participaram da sincinesia em 100%, 44,4% e 11,1% dos casos, respectivamente. Conclusões: Sincinesias oculares congênitas podem se apresentar de modo bastante eclético e incomum. A inervação aberrante presente em cada um desses casos os coloca na lista de candidatos a integrar o grupo das desordens congênitas da desenervação craniana.

A case of bilateral fourth nerve palsy associated with pseudotumor cerebri syndrome / Caso de paralisia bilateral de quarto nervo associada com síndrome do pseudotumor cerebral

ABSTRACT Pseudotumor cerebri syndrome is puzzling because it results in elevated intracranial pressure with no identifiable underlying cause. Ocular motor nerve palsies, other than a unilateral or bilateral sixth cranial nerve palsy, are rarely seen in patients with this condition. We report here on a 25-year-old female patient with bilateral fourth cranial nerve palsy caused by pseudotumor cerebri syndrome, whose ocular and systemic signs and symptoms of nerve palsy were completely resolved after medical treatment. We infer that fourth nerve palsy could be associated with pseudotumor cerebri syndrome; therefore, clinicians should consider rare ocular motor nerve palsies, even bilaterally, in patients with pseudotumor cerebri syndrome.

RESUMO A sindrome do pseudotumor cerebral é uma síndrome enigmática caracterizada por aumento da pressão intracraniana sem causa definida. Na síndrome do pseudotumor cerebral, as paralisias de nervo oculomotor além da paralisia unilateral ou bilateral do sexto nervo craniano são raramente vistas. Nós reportamos o caso de uma paciente feminina de 25 anos com paralisia bilateral de quarto nervo craniano como resultado da síndrome do pseudotumor cerebral. Após tratamento médico para síndrome do pseudotumor cerebral, os sinais e sintomas oculares e sistêmicos da paralisia nervosa foram resolvidos. Em conclusão, a paralisia de quarto nervo craniano pode estar associada com síndrome de pseudotumor cerebral, portanto médicos devem considerar paralisias raras de nervo oculomotor, mesmo bilateralmente, em pacientes com síndrome do pseudotumor cerebral.

Trochlear-oculomotor synkinesis: another congenital cranial dysinnervation disorder? / Sincinesia troclear-oculomotora: outro distúrbio congênito de denervação craniana?

ABSTRACT This report documents an unusual phenomenon. A 6-year-old girl with trochlear-oculomotor synkinesis presented with superior oblique and palpebral levator co-contraction. The literature was reviewed and the possibility of classifying this entity as a congenital cranial dysinnervation disorder was speculated.

RESUMO Este relato descreve um fenômeno incomum. Uma menina de 6 anos com sincinesia troclear-oculomotora apresentou co-contração do oblíquo superior e do levantador da pálpebra. A literatura foi revisada e especulou-se a possibilidade de classificar essa desordem como um distúrbio da congenital cranial dysinnervation disorder.

Rapid progression from trochlear nerve palsy to orbital apex syndrome as an initial presentation of advanced gastric cancer / 영남의대학술지

The most cases with orbital metastases have been reported in patients with a prior established diagnosis of cancer and widespread systemic involvement. However, ocular symptoms can be developed as an initial presentation of cancer in patients without cancer history. We report a case of rapid progression from trochlear nerve palsy to orbital apex syndrome as an initial presentation of advanced gastric cancer.

Delayed Trochlear Nerve Palsy Following Traumatic Subarachnoid Hemorrhage: Usefulness of High-Resolution Three Dimensional Magnetic Resonance Imaging and Unusual Course of the Nerve / 대한신경손상학회지

Cranial nerve palsies are relatively common after trauma, but trochlear nerve palsy is relatively uncommon. Although traumatic trochlear nerve palsy is easy to diagnose clinically because of extraocular movement disturbances, radiologic evaluations of this condition are difficult to perform because of the nerve's small size. Here, we report the case of a patient with delayed traumatic trochlear nerve palsy associated with a traumatic subarachnoid hemorrhage (SAH) and the related radiological findings, as obtained with high-resolution three-dimensional (3D) magnetic resonance imaging (MRI). A 63-year-old woman was brought to the emergency room after a minor head trauma. Neurologic examinations did not reveal any focal neurologic deficits. Brain computed tomography showed a traumatic SAH at the left ambient cistern. The patient complained of vertical diplopia at 3 days post-trauma. Ophthalmologic evaluations revealed trochlear nerve palsy on the left side. High-resolution 3D MRI, performed 20 days post-trauma, revealed continuity of the trochlear nerve and its abutted course by the posterior cerebral artery branch at the brain stem. Chemical irritation due to the SAH and the abutting nerve course were considered causative factors. The trochlear nerve palsy completely resolved during follow-up. This case shows the usefulness of high-resolution 3D MRI for evaluating trochlear nerve palsy.

Trochlear Nerve Palsy Caused by Quadrigeminal Cistern Lipoma

PURPOSE: To report a case of trochlear nerve palsy caused by quadrigeminal cistern lipoma located in the dorsal midbrain. CASE SUMMARY: A 65-year-old male visited our clinic for intermittent vertical diplopia over 2-year period. Symptoms of diplopia had worsened over the past two weeks. He had no previous medical history except having had diabetes for 1 month. The best-corrected visual acuity was 20/25 in the right eye and 20/20 in the left eye. Pupillary examination was not remarkable. Extraocular examination showed 4 prism diopters (PD) left hypertropia at distant gaze and 4 PD exotropia at near gaze, with adduction elevation of the left eye. The Bielschowsky head tilt test revealed 6 PD left hypertropia on the left gaze and orthotropia on the right tilt. Fundus examination showed excyclotorsion of the right eye and incyclotorsion of the left eye. Brain magnetic resonance imaging revealed quadrigeminal cistern lipoma. Prism glasses were prescribed to alleviate diplopia, and we followed up the lesions without further treatment. CONCLUSIONS: Trochlear nerve palsy can be caused by quadrigeminal cistern lipoma; however, it is uncommon for this condition to be caused by a compressive lesion. Prompt neuroimaging can be helpful to rule out the causes of this condition in patients with atypical symptoms.

Trochlear Nerve Schwannoma: Case Report and Literature Review

Schwannomas arise from the Schwann cells of the peripheral and cranial nerves. They represent 8% of the primary cerebral neoplasms. Although schwannomas usually develop in sensory nerves, most often on the vestibular and trigeminal nerves, in very rare cases they can develop in motor nerves. We reported an unusual case of a 29-year-old woman with headache, nausea, vomiting, and blurred vision ongoing for 3 years. Magnetic resonance of the brain showed a solid-cystic expanded injury, heterogeneous, with limits partially defined and epicenter on the pineal gland. The lesion presented hyposignal in T1 and isosignal in T2. An intense enhancement of the solid part was observed after contrast injection. Foci of calcification and absence of diffusion restriction were also observed. The patient underwent microneurosurgery with supracerebellar infratentorial approach in a seated position. Subtotal resection was performed with maintenance of calcified tumor tissue adhered to the right Rosenthal basal vein. In the postoperative phase, the patient remained with diplopia when looking down; however, she reported improvement of headache and nausea.

Os schwannomas surgem das células de Schwann, dos nervos periféricos e cranianos e representam 8% das neoplasias cerebrais primárias. Apesar de os schwannomas se desenvolverem comumente em nervos sensoriais, mais frequentemente no nervo vestibular e trigêmeo, em casos muito raros ele pode se desenvolver em nervos motores. Relatamos um caso raro, de uma paciente do sexo feminino, 29 anos, com quadro de cefaleia, náuseas, vômitos e turvação visual há três anos. Ressonância magnética de encéfalo demonstrou lesão expansiva sólido-cística, heterogênea, com limites parcialmente definidos e epicentro na glândula pineal. Apresentou hipossinal em T1 e isossinal em T2. Houve intenso realce da parte sólida após injeção de contraste. Foram observados focos de calcificação e ausência de restrição à difusão. A paciente foi submetida a tratamento microneurocirúrgico com acesso infratentorial supracerebelar em posição sentada. Houve ressecção subtotal com manutenção de tecido tumoral calcificado aderido a veia basal de Rosenthal direita. No pós-operatório, a paciente permaneceu com diplopia na mirada ocular para baixo, entretanto apresentou melhora de cefaleia e náuseas.

Imaging of Cranial Nerves III, IV, VI in Congenital Cranial Dysinnervation Disorders

Congenital cranial dysinnervation disorders are a group of diseases caused by abnormal development of cranial nerve nuclei or their axonal connections, resulting in aberrant innervation of the ocular and facial musculature. Its diagnosis could be facilitated by the development of high resolution thin-section magnetic resonance imaging. The purpose of this review is to describe the method to visualize cranial nerves III, IV, and VI and to present the imaging findings of congenital cranial dysinnervation disorders including congenital oculomotor nerve palsy, congenital trochlear nerve palsy, Duane retraction syndrome, Möbius syndrome, congenital fibrosis of the extraocular muscles, synergistic divergence, and synergistic convergence.

Curative effect of open reduction and internal fixation for capitellum and trochlea fractures / 中华创伤杂志

Objective To observe the curative effect of open reduction and internal fixation for capitellum and trochlea fractures.Methods A retrospective case series study was performed on 14 patients with capitellum and trochlea fractures without involving lateral and lateral column treated by open reduction and internal fixation from April 2008 to August 2016.There were four males and 10 females, aged from 24 to 62 years (mean, 41.4 years).Dubberley classification was type ⅠA in four patients, type ⅠB in three, type ⅡA in two, type ⅡB in one, type ⅢA in two, type ⅢB in two.Three patients were combined with ipsilateral capitulum radius fractures, and one patient with ipsilateral ulna olecranon fracture.Visual analogue scale (VAS) for elbow pain, elbow activity and Mayo elbow performance score (MEPS) were evaluated.Postoperative complications were also observed.Results All patients were followed up for 24-55 months (mean, 27.2 months).Fracture healed after (21.3±2.1)weeks.VAS was (2.1±1.9) points at 24 months postoperatively, with painless in three patients, mild pain in seven and moderate pain in four.Elbow flexion and extension angle was (93.4±15.2)°, elbow flexion angle was (114.3±11.1)°, elbow extension angle was (33.6±20.7)°, forearm front rotation angle was (75.2±12.5)°, and forearm back rotation angle was (83.2±8.2)°.No patients had elbow instability, ischemic necrosis or internal fixation loosening.MEPS was (74.2±17.8)points, including excellent results in 3 cases, good in 8, fair in 2 and poor in 1.One case of Dubberley type ⅢA developed mild ulnar nerve symptom and alleviated after 3 months.Five cases (two type ⅢA, one type ⅢB, one type ⅡA, one type ⅡB) had a small amount of heterotopic ossification 6 month after operation, but elbow flexion and extension activity was good.One case of Dubberley type ⅢB combined with fracture of the olecranon process had elbow heterotopic ossification 1 month after operation, further developed large displaced ossification around the elbow 6 months after operation, and showed elbow flexion and extension of 30° 2 years after operation that was significantly limited compared to the contralateral side.Conclusion Open reduction and internal fixation of capitellum and trochlea fractures can improve elbow activity and reduce incidence of postoperative complication.

A Case of Trochlear Nerve Schwannoma Presenting with Binocular Diplopia

PURPOSE: To report a case of unilateral trochlear nerve schwannoma in a patient without neurofibromatosis. CASE SUMMARY: A 58-year-old male presented with acute onset of diplopia which developed 10 days prior. Alternate prism cover test, ductions and versions and Bielschowsky three-step test were compatible with left superior oblique muscle palsy. High-resolution magnetic resonance imaging showed a 6-mm-sized lobulated mass in the cisternal segment of the left trochlear nerve passing lateral to the brainstem. An additional thin-section gadolinium-enhanced orbit magnetic resonance imaging showed definite enhancement in the entire portion of the lobulated mass, compatible with a trochlear nerve schwannoma. Diplopia was managed conservatively with prism glasses and regular follow-up examinations were recommended without further treatment. CONCLUSIONS: A trochlear nerve tumor should be considered in adults who develop diplopia associated with acquired superior oblique muscle palsy.

Skew deviation e ocular tilt reaction versus paralisia do nervo troclear / Skew deviation and ocular tilt reaction versus trochlear nerve palsy

Skew deviation é o desalinhamento vertical dos olhos causado por disfunção unilateral no tronco cerebral, cerebelo ou sistema vestibular periférico, comprometendo as vias por onde transitam impulsos dos órgãos otolíticos aos núcleos oculomotores. Está frequentemente associado a torção ocular e inclinação da cabeça, compondo, assim, a ocular tilt reaction. Neste artigo os autores ressaltam aspectos históricos, fisiológicos, etiopatogênicos e clínicos que contribuem para caracterizar ambas as condições, além da diferenciação clínica com a paralisia do nervo troclear.

Skew deviation is the vertical misalignment of the eyes caused by unilateral dysfunction in the brain stem, cerebellum or peripheral vestibular system, resulting imbalance in otolith input to the oculo-motor system. It's often associated with ocular torsion and head tilt, which together comprise the ocular tilt reaction. In this article the authors emphasize the historical, physiological, etiopathogenesis and clinical aspects that contribute to characterize both conditions, and help to differentiate them clinically from trochlear nerve palsy.

Modifications on Dorsum of Neck of Talus (Squatting Facets and Trochlear Extensions) in Indians.

Objective: Habitual squatting in humans is associated with modifi cations of ankle especially the neck of the talus (squatting facets) and its trochlear surface (trochlear extensions) that characterize the strong pressure and traction forces on ankle joints in state of hyperdorsifl exion. Present study was done to fi nd out variations and incidences of various types of modifi cations of neck of talus thoroughly and to determine regional peculiarities of these modifi cations in Indians. Material and Methods: 300 dry (150 right and 150 left) adult tali were taken for present study. Each talus was examined for the presence of various patterns of articular facets on neck of talus and extensions of its trochlear surface. Statistical analysis of data was performed by using Fisher exact test with 95% confi dence limits. Results: Lateral squatting facet was found in 136 tali (45.3%). Incidences of medial, combined & continuous gutter like squatting facets were 7.7%, 3.3% & 4.3% respectively. Lateral and medial extensions of trochlear surface were found in 22.3% and 23.7% respectively. Conclusions: Modifi cations of the neck of talus (squatting facets and trochlear extensions) are result of prolonged squatting positions which is common habit of Indian population and incidences of these variations can be used as an anthropological marker for racial and regional diff erentiation of unidentifi ed bones.

A Case of Bilateral Trochlear Nerve Palsy Following Cisternography

PURPOSE: To report a case of bilateral trochlear nerve palsy following cisternography. CASE SUMMARY: A 43-year-old male with intermittent watery rhinorrhea persisting for 3 months visited the neurosurgery department of our institute. His past medical history included removal of a pituitary adenoma 22 years prior to presentation. Cerebrospinal fluid leakage was suspected and cisternography was performed. The patient was referred to our ophthalmology department for diplopia 3 days after the cisternography. An alternate prism cover test showed 5 prism diopter (PD) right hypertrophia in the primary position, and underaction of bilateral superior oblique muscles and overaction of the left inferior oblique muscle. A positive Bielschowsky test with the head tilted to either side was observed and excyclotorsion was 9degrees on the double Maddox rod test. The patient was diagnosed with bilateral trochlear nerve palsy. After 2 years of follow-up, diplopia persisted and recession of the bilateral inferior oblique muscles was performed. After the surgery, diplopia disappeared, the fundus photography showed no excyclotorsion, and the double Maddox rod test indicated 3degrees of excyclotorsion. CONCLUSIONS: Cisternography should be carefully performed due to the possibility of bilateral trochlear nerve palsy, an extremely rare but possible occurrence following the procedure.

Occurrence of Trochlear Nerve Palsy after Epiduroscopic Laser Discectomy and Neural Decompression

Epiduroscopic laser discectomy and neural decompression (ELND) is known as an effective treatment for intractable lumbar pain and radiating pain which develop after lumbar surgery, as well as for herniation of the intervertebral disk and spinal stenosis. However, various complications occur due to the invasiveness of this procedure and epidural adhesion, and rarely, cranial nerve damage can occur due to increased intracranial pressure. Here, the authors report case in which double vision occurred after epiduroscopic laser discectomy and neural decompression in a patient with failed back surgery syndrome (FBSS).

Clinical Features and Natural Course of Superior Oblique Palsy

PURPOSE: To evaluate the natural course of superior oblique palsy (SOP) with objective criteria, and to show the contemporary etiology and recovery rates among several factors. The clinical features of SOP were compared to previous studies. METHODS: A retrospective chart review of 80 patients diagnosed with SOP between January 1, 2006 and December 31, 2011 was performed. RESULTS: Clinical SOP features showed variation when compared to previous studies. Out of 80 patients, 71 were identified with unilateral isolated and 9 bilateral cases of SOP. Twenty cases were congenital and 60 cases were acquired SOPs. Acquired SOPs were affected most commonly by trauma (31%) and vascular disease (30%). Twenty-four out of 49 patients, who were followed up over 2 months after the first visit recovered, especially vascular origin cases, which was statistically significant (75%, p = 0.000). Patients with initial vertical deviation smaller than 5 Prism diopters (PD) experienced a more successful recovery than patients with an initial deviation larger than 5 PD. CONCLUSIONS: SOP has different recovery rates depending on the etiology. Accurate ocular examination and understanding of SOP etiology are necessary for successful treatment.

A Case of Herpes Zoster Ophthalmicus with Isolated Trochlear Nerve Involvement

BACKGROUND: Herpes zoster ophthalmicus (HZO) can involve the oculomotor nerve; however, isolated trochlear nerve palsy has rarely been reported. CASE REPORT: An 83-year-old man who suffered from HZO in the right frontal area and scalp subsequently developed vertical diplopia and severe pain. Cerebrospinal fluid examination and brain MRI revealed no abnormalities. Isolated right trochlear nerve palsy was diagnosed based on the findings of neuro-ophthalmological tests. CONCLUSIONS: Isolated trochlear nerve involvement associated with HZO is very rare and may be easily overlooked. Physicians should carefully examine oculomotor involvement in HZO.

A Case of Neurosyphilis with Acute Optic Neuritis and Trochlear Nerve Palsy in Human Immunodeficiency Virus Infected Male

With the increase in human immunodeficiency virus (HIV) infection, the incidence of syphilis also increased worldwide presenting with diverse clinical manifestations. We experienced a case of symptomatic early neurosyphilis manifesting as optic neuritis and trochlear nerve palsy with typical skin lesions of secondary syphilis in an HIV-Infected patient. Intravenous penicillin and oral steroid were administered for 2 weeks. Skin lesions improved after a week, and ocular lesions resolved completely after eight weeks of treatment.

Isolated, Contralateral Trochlear Nerve Palsy Associated with a Ruptured Right Posterior Communicating Artery Aneurysm

Trochlear nerve palsy associated with spontaneous subarachnoid hemorrhage (SAH) is known to be a rare malady. We report here on a patient who suffered with left trochlear nerve palsy following rupture of a right posterior communicating artery aneurysm. A 56-year-woman visited our emergency department with stuporous mental change. Her Hunt-and-Hess grade was 3 and the Fisher grade was 4. Cerebral angiography revealed a ruptured aneurysm of the right posterior communicating artery. The aneurysm was clipped via a right pterional approach on the day of admission. The patient complained of diplopia when she gazed to the left side, and the ophthalmologist found limited left inferolateral side gazing due to left superior oblique muscle palsy on day 3. Elevated intracranial pressure, intraventricular hemorrhage or a dense clot in the basal cisterns might have caused this trochlear nerve palsy.

Ocular motor nerve palsy in head injury patients / 国际眼科杂志(Guoji Yanke Zazhi)

AIM: To determine ocular motor nerve palsy in patients with head trauma.METHODS: There were three hundred admitted cases of head trauma in neurosurgery department of Shahid Rahnemon Hospital from March 2006 to September 2006. Data were collected with ophthalmic examinations, filled in questionnaires and analyzed by SPSS software statistically including Chi-square test and Fisher's exact test.RESULTS: A total of 300 patients were reviewed, 242 (81.1%) men and 58 (18.9%) women. Their age ranged from 1 to 87 years (mean of 46 years). Accident and fall were the most common causes of head trauma, occurring in 247 (82.3%) patients and most of patients had Glasgow Coma Scale (GCS) 13-15(82.3%). The most cerebral lesion was subdural and subarachnoid hematoma. Isolated trochlear and abducens nerve palsy (28.6%), isolated oculomotor nerve and combined nerve palsies (combination of 3rd and 4th cranial nerves, 14.3%) were the most affected nerves.CONCLUSION: Although the cranial nerve palsy is rare with minor head trauma, according to their observation in emergency room, neuroophtalmic examination is advised.

Transient Isolated Trochlear Nerve Palsy Associated with Rathke's Cleft Cyst

We report the case of a 52-year-old woman who developed vertical diplopia of 1-days duration. Neuro-ophthalmological testing revealed left trochlear nerve palsy, and sellar MRI revealed a 1.5 cm-sized pituitary mass lesion, a Rathke's cleft cyst. The diplopia disappeared spontaneously after 6 days.